Long‐term results in children with head and neck rhabdomyosarcoma: A report from the Italian Soft Tissue Sarcoma Committee

Affinita, Maria Carmen; Ferrari, Andrea; Milano, Giuseppe Maria; Scarzello, Giovanni; Leonardis, Francesco De; Coccoli, Luca; Pericoli, Roberta; Basso, Eleonora; Zanetti, Ilaria; Scagnellato, Angela; Bisogno, Gianni

doi:10.1002/pbc.26876

Cited by 18 publications

(16 citation statements)

References 14 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The four histological subtypes of RMS currently recognized by the World Health Organization include embryonal, alveolar, pleomorphic, and spindle cell/sclerosing 4 . While RMS can arise throughout the body, the most common tumor sites are within the head and neck (35–40%) 1,3 . Among head and neck RMS (HNRMS), lesions may be orbital (25%), parameningeal (PM; 50%), or nonparameningeal (non‐PM; 25%; Table I).…”

Section: Introductionmentioning

confidence: 99%

“…RMS affects only 4.5 out of 1 million children in the United States 9 . Due to its rarity, few institutions have extensive experience with HNRMS, and most single‐institution studies report a relatively small number of patients 1–3,10,11 . The purpose of this study is to analyze the experience of a multidisciplinary team at Boston Children's Hospital and Dana‐Farber Cancer Institute with the evaluation and management of HNRMS in children.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Role of Surgery in Rhabdomyosarcoma of the Head and Neck in Children

et al. 2020

View full text Add to dashboard Cite

Objectives Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children. The goal of this research is to analyze the role of surgery in the management of pediatric parameningeal (PM) and non‐PM head and neck RMS (HNRMS). Study Design Retrospective review. Methods Retrospective chart review of patients <20 years of age treated for HNRMS between 1970 and 2015. Clinical presentation, tumor characteristics, treatment, recurrence, follow‐up, and outcome data were collected. Results Of 97 patients with HNRMS, 56% were male. Overall median (IQR: interquartile range) age at diagnosis was 5.8 (3.3–9.8) years. Sixty‐five patients (67%) had PM tumors. Of 75 patients with histologic subtype identified, 51 (53%) had embryonal and 20 (21%) alveolar RMS. Almost all patients received chemotherapy (99%) and radiotherapy (95%). Forty‐four patients (45%) underwent surgery. Surgery was more likely to be conducted in patients with lesions of a non‐PM site. Median follow‐up time was 3.4 years (IQR: 1.1–10.8). In 5 years of follow‐up, 20% (17 of 85) died and 29% (20 of 70) had recurrence. The estimated 5‐year survival rate was 72% (95% CI, 57.8, 81.5%). Surgery was associated with a reduced risk of mortality after accounting for TNM stage 4 and tumor site (adjusted HR 0.24; 95% CI, 0.07, 0.79; P = .02). The association between surgery and risk of mortality was similar in PM and non‐PM tumors. Conclusion A multimodal protocol for treatment including chemotherapy, surgery, and radiotherapy is the mainstay for management of children with HNRMS. While surgery is more commonly used to treat non‐PM HNRMS, patients who are able to undergo surgery have significantly higher 5‐year survival. Level of Evidence 4 Laryngoscope, 131:E984–E992, 2021

show abstract

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Role of Surgery in Rhabdomyosarcoma of the Head and Neck in Children

et al. 2020

View full text Add to dashboard Cite

show abstract

“…orbital location with bone infiltration, epidural, limb, bladder, or prostate tumours), tumour size greater than 5 cm, age over 10 years, metastases to the lymph nodes, and distant metastases. Although RMS in children is an aggressive sarcoma, the survival results are 75% due to combination therapy [24].…”

Section: Prognostic Factors and Treatment Resultsmentioning

confidence: 99%

“…The use of radiotherapy also dependents on the patient's age. The five-year survival rate in patients with RMS is 50-75%, with the prognosis depending on the stage and age of the patient [24].…”

Section: Rhabdomyosarcomamentioning

confidence: 99%

Recommendations in management of head and neck sarcomas

et al. 2019

View full text Add to dashboard Cite

Sarcomas of the head and neck are rare malignant tumours with incidence 2% of head and neck cancers, 30% of all sarcomas in children and 4-10% of sarcomas in adults. The most common sarcoma in children is rhabdomyosarcoma (RMS) and Ewing sarcoma (ES), in contrary in elderlies there is angiosarcoma; osteosarcoma and chondrosarcoma are the most common bone tumours in head and neck. The typical symptoms are: painless tumour in the head and neck region, difficulty with speaking, hoarseness, dysphagia, nasal obturation, or dysfunction of cranial nerves. The key point of diagnostics is magnetic resonance and computed tomography of the primary tumour. The treatment of patients with sarcoma should be carried out in referral centers, where experienced multidisciplinary team proceed the surgical removal of a primary tumour with reconstructive surgery as well as perioperative systemic therapy (in selected cases). The recommendations were developed by the Head and Neck Sarcoma Group of the Polish Registry of Bone Tumors.

show abstract

“…These symptoms are usually easier to detect early than the symptoms of parameningeal RMS, which are unspecific and misleading for a long time [14]. Accordingly, the prognosis in children with non-parameningeal RMS is better than for parameningeal RMS (10-year overall survival 74.2% vs. 66.1%) [15,16].…”

Section: Discussionmentioning

confidence: 99%

Misdiagnosis of a chin abscess in a teenager with rhabdomyosarcoma – consequences for the patient’s health and quality of life

Krawczyk¹,

Bień²

2020

polp

View full text Add to dashboard Cite

Childhood rhabdomyosarcoma (RMS) develops frequently within head and neck (H&N) structures. Its clinical manifestation may mimic more common benign, inflammatory, and congenital lesions. A 16-year-old boy with painless purple swelling of the chin soft tissues was treated with local and systemic antibiotics. Because no improvement was achieved, the growing "abscess" was incised and drained many times in the surgical department. After three months of ineffective therapies, the patient was transferred to the clinical hospital, where a tumour infiltrating the chin and oral cavity floor, impairing speech and swallowing, was detected. Alveolar RMS was diagnosed by histopathology of the tumour biopsy. Due to unfavourable prognostic factors, the patient was classified to a very high-risk group and received aggressive long-lasting oncological treatment. Although he achieved full remission, the sequelae of therapy affect his quality of life. Despite its rarity, RMS should be included in the differential diagnosis of H&N lesions, especially painless and rapidly growing.

show abstract

Long‐term results in children with head and neck rhabdomyosarcoma: A report from the Italian Soft Tissue Sarcoma Committee

Cited by 18 publications

References 14 publications

Role of Surgery in Rhabdomyosarcoma of the Head and Neck in Children

Role of Surgery in Rhabdomyosarcoma of the Head and Neck in Children

Recommendations in management of head and neck sarcomas

Misdiagnosis of a chin abscess in a teenager with rhabdomyosarcoma – consequences for the patient’s health and quality of life

Contact Info

Product

Resources

About